Werewolf syndrome, Ambras syndrome or Hypertrichosis

This is an extremely rare condition with excessive hair growth over and above the normal for the age, gender and race of an individual.It can be generalized or localized, congenital or acquired.

Werewolf Syndrome (Picture Credit; reddit)

First documented by Aldrovandus In 1648

The gender and racial prevalence for this condition is not yet known as Less than 100 cases have been reported worldwide, with an incidence of about 1 in a billion to 1 in 10 billion (congenital hypertrichosis lanuginosa).
It is apparent at birth (depending on the type).

Hypertrichosis differs from Hirsutism in that, it is not androgen related and does not follow the male pattern of Hair distribution, while Hirsutism commonly occurs in women and presents as androgen-induced male pattern hair growth.

Classification
Congenital
• Hypertrichosis lanuginosa: completely covered in thin lanugo hair; long, silky and light colored hairs
• Generalised hypertrichosis: involves vellus and terminal hairs.
• Terminal hypertrichosis:  coarser and thicker hairs and usually pigmented, accompanied by gingival hyperplasia.
• Circumscribed hypertrichosis; presence of thick vellus hair on the upper extremities ( e.g hairy eyebrows )
• Localised hypertrichosis,
• Nevoid hypertrichosis; excessive terminal hair and is usually not related to any diseases.

Acquired
• Hypertrichosis lanuginosa; fine and unpigmented hair on the face, trunk and armpit.
• Generalised hypertrichosis:
• Patterned hypertrichosis: increase in hair growth in a pattern formation
• Localised hypertrichosis: increase in hair density and length often secondary to irritation or trauma



Causes
Congenital
Genetically acquired; autosomal dominant mutation on 8q (Hypertrichosis lanuginosa),autosomal dominant mutation on chromosome x24-q27.1 (Generalized hypertrichosis), mutation in MAP2K6 on chromosome 17 (Terminal hypertrichosis).

Acquired

• Cancer,
• Endocrinopathies; hyperthyroidism
• Metabolic disorders such as porphyria cutanea tarda
• Anorexia nervosa
• Medications; iodine, psoralens, minoxidil, steroids,cyclosporine, diazoxide,phenytoin, streptomycin, hexachlorobenzene, penicillamine, heavy metals, sodiumtetradecyl sulfate, acetazolamide, and interferon.
•  Minor abrasions or injury requiring a plaster cast
• Longstanding lichen simplex, infections and vaccinations.
• Head injuries, malnutrition, starvation and AIDS (are less common causes)

Diagnosis
 This is mainly clinical, however, hair biopsy for histologic classification is indicated; Lanugo hairs tend to be non-pigmented,  Vellus hair may be pigmented or nonpigmented. Lanugo and vellus hairs can be difficult to distinguish at histological examination.

Differential diagnosis
Hirsutism
Mucopolysaccharidoses Types I-VII
Rubinstein-Taybi Syndrome


Treatment
Drugs; Eflornithine (Vaniqa cream), Has been found to be very useful.
           Bleaching can improve congenital hypertrichosis lanuginosa (CHL) patients’ appearance.
           Antidepressants can be used in patients with depression.
Physical depilation; tweezing or plucking, shaving, and waxing. Tweezing is an effective temporary hair removal method, but it is a slow, tedious, and painful process.
Chemical depilatories; contain sulfides, thioglycolates, and enzymatic depilatory agents. They break down hair by cleaving its cysteine linkages thereby causing minimal damage to the underlying skin. Electrolysis,
Intense pulsed light therapy
Diathermy and Radiation therapy can be used rarely

Hypertrichosis (Picture Credit; real world image.)

Surgical Care
    Laser hair removal has been proposed as a treatment option, although it is still remains controversial.


NOTES

1. Beighton P. Congenital hypertrichosis lanuginosa. Arch Dermatol. Jun 1970;101(6):669-72.

2. Klein  AWRish  DC Depilatory and shaving products. Clin Dermatol. 1988;668- 70

3. Lesiewicz  JGoldsmith  LA Antizyme release is an early event in ornithine decarboxylase induction by hair plucking. J Invest Dermatol. 1983;8097- 100

4. Hynd  PINancarrow  MJ Inhibition of polyamine synthesis alters hair follicle function and fiber composition. J Invest Dermatol. 1996;106249- 253

5. Medical Dictionary; Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved

6. Medscape; eMedicine.

7. Partridge JW. Congenital hypertrichosis lanuginosa: neonatal shaving. Arch Dis Child. Jun 1987;62(6):623-5

8. Tadin M, Braverman E, Cianfarani S, et al. Complex cytogenetic rearrangement of chromosome 8q in a case of Ambras syndrome. Am J Med Genet. Jul 22 2001;102(1):100-4.

9. Wikipedia | hypertrichosis